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Lookup NU author(s): Dr Ben ShillitoeORCiD, Dr Catherine Stroud, Dr Terence Flood, Professor Mark PearceORCiD, Professor Andrew GenneryORCiD
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2026 Shillitoe et al. Due to limitations in current therapies (immunoglobulin replacement therapy), complications in X-linked agammaglobulinemia (XLA) such as bronchiectasis may continue to occur, with subsequent significant impacts on healthrelated quality of life (HRQoL). There were no significant differences in PedsQL 4.0 total scores against UK healthy norms (self 80.98, P = 0.277; parent 79.76, P = 0.465). There were no significant differences in the SF36v2 physical component score (PCS) against UK healthy norms (49.10, P = 0.712). However, XLA patients with bronchiectasis had significantly worse PCS than patients without (47.88 versus 55.14, P < 0.001) and significantly worse PCS than UK healthy norms (P = 0.004). In the absence of bronchiectasis, HRQoL is comparable to UK healthy norms. However, XLA patients with bronchiectasis have significantly worse HRQoL than patients without bronchiectasis and UK healthy norms. These data demonstrate that further work into novel therapies are needed to prevent bronchiectasis to enable XLA patients to have a normal quality of life.
Author(s): Shillitoe B, Bourne H, Stroud C, Flood T, Buckland M, Ip W, Worth A, Hackett S, Herwadkar A, Coulter T, Jolles S, Garcez T, Moya E, Faust SN, Pearce MS, Gennery AR
Publication type: Article
Publication status: Published
Journal: Journal of Human Immunity
Year: 2026
Volume: 2
Issue: 3
Print publication date: 04/05/2026
Online publication date: 19/02/2026
Acceptance date: 06/02/2026
Date deposited: 01/06/2026
ISSN (electronic): 3065-8993
Publisher: Rockefeller University Press
URL: https://doi.org/10.70962/jhi.20250198
DOI: 10.70962/jhi.20250198
Data Access Statement: All relevant data that can be shared can be found in supplemental material. Additional data are available from the corresponding author upon reasonable request from health care professionals and within national General Data Protection Regulation rules and regulations.
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